For instance, a phase II clinical trial aimed to test the safety and effectiveness of the combination of nivolumab (anti-PD-1 antibody) and ipilimumab (anti-CTLA-4 antibody) in SMARCB1/INI1-negative tumors is ongoing (NCT04416568) and a phase I/II study to assess the value of combining these immune checkpoint inhibitors (nivolumab and ipilimumab) with tazemetostat has just been launched (NCT05407441) for the treatment of ES, MRT, AT/RT, chordoma and other SMARCB1/INI1- or SMARCA4-deficient tumors. This evidence concerns the gene SMARCB1 and chordoma.